Iron overload in myelodysplastic syndromes (MDS).

Iron overload in myelodysplastic syndromes (MDS). Int J Hematol. 2017 Nov 25;: Authors: Gattermann N Abstract Iron overload (IOL) starts to develop in MDS patients before they become transfusion-dependent because ineffective erythropoiesis suppresses hepcidin production in the liver and thus leads to unrestrained intestinal iron uptake. However, the most important cause of iron overload in MDS is chronic transfusion therapy. While transfusion dependency by itself is a negative prognostic factor reflecting poor bone marrow function, the ensuing transfusional iron overload has an additional dose-dependent negative impact on the survival of patients with lower risk MDS. Cardiac dysfunction appears to be important in this context, as a consequence of chronic anemia, age-related cardiac comorbidity, and iron overload. Another potential problem is iron-related endothelial dysfunction. There is some evidence that with increasing age, high circulating iron levels worsen the atherosclerotic phenotype. Transfusional IOL also appears to aggravate bone marrow failure in MDS, through unfavorable effects on mesenchymal stromal cells as well a hematopoietic cells, particularly erythroid precursors. Patient series and clinical trials have shown that the iron chelators deferoxamine and deferasirox can improve hematopoiesis in a minority of transfusion-dependent patients. Analyses of registry data suggest that iron chelation provides a survival benefit for patients with M...
Source: International Journal of Hematology - Category: Hematology Authors: Tags: Int J Hematol Source Type: research

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CONCLUSIONS: HS-FCM can sensitively detect minor PNH clones and reduce false-positive C-FCM minor PNH clone cases in AA/low-grade MDS patients. PMID: 30430777 [PubMed - in process]
Source: Annals of Laboratory Medicine - Category: Laboratory Medicine Tags: Ann Lab Med Source Type: research
Publication date: Available online 7 November 2018Source: Blood Cells, Molecules, and DiseasesAuthor(s): Jin-jun Hu, Jing Xu, Ting-ting Tian, Juan Xie, Li-fang Fan, Guiyang Zhu, Ting Xia, Xi Chen, Yan-hong Tan, Xiu-hua Chen, Fanggang Ren, Yao-fang Zhang, Hong-wei Wang, Zhi-fang XuAbstractTo study the association between TET2rs2454206, TET2rs12498609 and ASXL1rs3746609 and Myelodysplastic syndromes (MDS), a total of 90 MDS patients and 143 healthy volunteers were included. The clinical data, bone marrow samples of patients and peripheral blood samples of volunteers were obtained. We found TET2rs2454206 G/A genotype, TET2rs1...
Source: Blood Cells, Molecules, and Diseases - Category: Hematology Source Type: research
Purpose of review Anaemia is a common haematological presentation in patients with bone marrow failure, yet a challenging condition to treat. As anaemia has a direct impact on the patient's symptoms, managing anaemia in the common bone marrow failure conditions, such as myelodysplastic syndrome will help to improve the quality of life. This review discusses the available treatment options and the benefit of improving the haemoglobin level. Recent findings Managing anaemia effectively has shown to improve the patient outcome, yet treatment option remain limited. Recently, activin inhibitors such as Luspatercept have sh...
Source: Current Opinion in Supportive and Palliative Care - Category: Palliative Care Tags: BLOOD, BONE MARROW AND LYMPHATICS: Edited by Christopher Dalley Source Type: research
AbstractMinor populations of glycosylphosphatidylinositol-anchored protein-deficient (GPI[ −]) cells in the peripheral blood may have a prognostic value in bone marrow failure (BMF). Our objective is to establish the optimal flow cytometry (FCM) assay that can discriminate GPI(−) populations specific to BMF from those of healthy individuals. To identify a cut-off that discriminates GP I(−) rare cells from GPI(+) cells, we determined a position of the borderline that separates the GPI(−) from GPI(+) cells on a scattergram by testing more than 30 healthy individuals, such that no GPI(−) dot fell...
Source: Annals of Hematology - Category: Hematology Source Type: research
Abstract Splicing factor gene mutations are found in 60-70% of patients with myelodysplastic syndromes (MDS). We investigated the effects of splicing factor gene mutations on the diagnosis, patient characteristics, and prognosis of MDS. A total of 106 patients with MDS were included. The percentage of patients with MDS with ring sideroblasts (14.15%) as per the 2017 WHO classification was significantly higher than that of patients with refractory anemia with ring sideroblasts (2.88%) as per the 2008 WHO classification (P = 0.005). Splicing factor mutations were detected in 32 patients (13 SF3B1, 8 U2...
Source: International Journal of Hematology - Category: Hematology Authors: Tags: Int J Hematol Source Type: research
Authors: Sachiyo O, Masahiro T, Tsutomu T, Makoto I, Yutaka H, Nobumasa M, Takamichi K, Nozomi O, Shinpei M, Kazuhiro T, Yusuke K, Masanori O, Yusuke K, Yasuhisa H, Kazuo H, Yasumasa N Abstract Fanconi anemia (FA) is a disorder of chromosomal fragility characterized by progression to aplastic anemia, myelodysplastic syndrome, and leukemia. FA patients are also predisposed to solid cancers. A case of FA in an adult patient who developed tongue and superficial esophageal cancers following hematopoietic stem cell transplantation is reported. This case was considered significant because it is the first reported case of...
Source: Internal Medicine - Category: Internal Medicine Tags: Intern Med Source Type: research
Conclusions: G-CSF for patients with AA is not associated with a higher occurrence of secondary malignant neoplasm, mainly MDS/AML, or PNH.Acta Haematol 2018;140:141 –145
Source: Acta Haematologica - Category: Hematology Source Type: research
International Journal of Laboratory Hematology, EarlyView.
Source: Clinical and Laboratory Haematology - Category: Hematology Authors: Source Type: research
CONCLUSIONS: Our prospective, four-step approach is an efficient and logical strategy to facilitate the diagnosis of MDS on the basis of unexplained anemia and/or macrocytosis, and may allow the early diagnosis of the most serious causes of anemia. Molecular analysis of genes related to MDS could be a promising diagnostic and prognostic approach. PMID: 30290085 [PubMed - as supplied by publisher]
Source: International Journal of Laboratory Hematology - Category: Hematology Authors: Tags: Int J Lab Hematol Source Type: research
, Rose C, Lachenal F, Toma A, Pica GM, Carre M, Garban F, Mariette C, Cahn JY, Meunier M, Herault O, Fenaux P, Wagner-Ballon O, Bardet V, Dreyfus F, Fontenay M Abstract Erythropoiesis-stimulating agents are generally the first line of treatment of anemia in lower risk myelodysplastic syndrome patients. We prospectively investigated the predictive value of somatic mutations, and biomarkers of ineffective erythropoiesis including flow cytometry RED score, serum GDF-15, and hepcidin levels. Inclusion criteria were: Erythropoiesis stimulating agents naive, IPSS low or intermediate-1 MDS with Hemoglobin level4 (p=0.05)...
Source: Haematologica - Category: Hematology Authors: Tags: Haematologica Source Type: research
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